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Immunology
Rx Review R3 Immunology and Autoimmune Diseases
| Question | Answer |
|---|---|
| Another name for Berger Disease: | IgA Nephropathy |
| What is the MCC of GROSS HEMATURIA secondary to Primary glomerulonephritis? | IgA Nephropathy |
| What is the IM findings of Berger Disease? | Non-linear mesangial deposits of IgA |
| MPGN type 2 is considered both: | Nephrotic and Nephritic Syndrome |
| What is the IM findings of MPGN type 2? | Dense deposits within basement membrane |
| Podocyte effacement | MCD |
| MCD is often associated to: | Hematologic malignancies, such as Hodgkin Lymphoma |
| SLE IM description: | Subendothelial deposits, composed primarily of anti-dsDNA bound to DNA |
| Which organ are acute phase reactants made? | Liver |
| Acute phase reactants act as: | Opsonins, and further increase the production of cytokines |
| What is Ataxia-Telangiectasia MCC? | Defective DNA repair mechanisms |
| What is the triad of symptoms of Ataxia-Telangiectasia? | 1. Ataxia ---> frequent falls 2. Spiderangionmas 3. IgA deficiency --> recurrent sinopulmonary infections |
| What is the triad of Wiskott-Aldrich Syndrome (WAS)? | 1. Eczema 2. Thrombocytopenia 3. Recurrent infections |
| X-linked Combined Immunodeficiency disorder seen with Eczema? | Wiskott-Aldrich Syndrome |
| Which immunoglobulins are elevated in WAS? | IgE and IgA |
| What is meant with Combined Immunodeficiency? | B-cell and T-cell deficiency |
| Non-inflamed (cold) skin abscesses are often key in the presentation of ______________________________. | Job Syndrome (Hyper-IgE syndrome) |
| What is another name for Job syndrome? | Hyper-IgE syndrome |
| Underdeveloped Paracortex of the lymph node. Dx? | DiGeorge Syndrome |
| What immunodeficiency is seen with underdeveloped Cortical follicles of the lymph node? | X-linked agammaglobulinemia |
| What is the most common form of arthritis in children? | Juvenile Idiopathic Arthritis |
| What are the key characteristics of Juvenile Idiopathic Arthritis (JIA)? | - Quotidian fevers - Arthritis - Classic Salmon pink rash |
| Salmon pink rash in thorax + pain in the wrist + recurrent subsiding fevers in a 16 year old female. Dx? | Juvenile Idiopathic Arthritis |
| Which conditions yield a positive PPD test? | 1. TB 2. Erythema nodosum 3. Contact dermatitis 4. Hashimoto's thyroiditis 5. Multiple Sclerosis |
| What are possible complications of Rh Incompatibility? | 1. Hydrops fetalis 2. Hemolytic Disease of the Newborn |
| What is the MC treatment for Rh Incompatibility? | Exchange transfusion with a matched Rh (-) blood |
| What is a common immunologic function of EOSINOPHILS? | Fight off parasitic infections |
| What is the type of immunity elicited by Eosinophils in the attack against parasitic infections? | Antibody-dependent cell-mediated cytotoxicity |
| Associated released protein form Eosinophils? | MBP (Major Basic Protein) |
| Occlusion of graft vessels, causing ischemia and necrosis. Describes which type of tissue rejection? | Hyperacute |
| Vasculitis of graft vessels is seen in ______________ rejection | Acute |
| Chronic Rejection is seen with: | Fibrosis of graft tissue and blood vessels |
| What are common clinical findings in GVHD? | Dermatitis, Jaundice, Diarrhea, and hepatosplenomegaly. |
| Person after a transplant presents 32 days after event with skin rash, enlarged liver, elevated ALT/AST, and severe diarrhea. Dx? | GVHD (Graft vs Host Disease) |
| What tissues or organs are the most common to develop GVHD? | Allogenic blood and Bone marrow |
| What is the pathophysiology of GVHD? | Donor lymphocytes reacting against major or minor histocompatibility antigens on recipient cells. |
| Decrease in IgA, IgE and IgM is seen with ________________________. | X-linked agammaglobulinemia (XLA) |
| Defect in gene coding for Bruton Tyrosine Kinase | X-linked agammaglobulinemia (XLA) |
| What gene is affected in XLA? | Bruton Tyrosine Kinase |
| What is the main function of BTK? | B-cell development and its absence increases risk for bacterial infections |
| What is the MC treatment for Bruton Agammaglobulinemia? | Intramuscular gamma-globulin injections |
| What kind of person description is often used in XLA vignette? | Young male with recurrent infections with Encapsulated organisms. |
| INF-alpha is used to treat? | 1. IL-12 deficiency 2. Chronic Granolomatous disease |
| Filgrastim : | Functions as stimulating factor to increase the number of neutrophils. |
| What drug or medication is used to elevate or increase the amount of neutrophils in a patient? | Filgrastim |
| What is a common location of IgA? | Breast milk and Peyer's patches in the small intestine |
| Where in the Small Intestine is IgA found? | Peyer's patches from the lamina propria and the submucosa |
| What immunoglobulin has the highest concentration in tissue and blood? | IgG |
| IgG is able to: | Fix complement and cross the placenta |
| IgM is able to: | Fix complement |
| Which immunoglobulin is able to fix complement but CANNOT cross the placenta? | IgM |
| What is the main Ig of Humoral immunity? | IgM |
| Igm is found mainly in the _______________. | Serum |
| What kind of infection present in patient should raise suspicion of IL-12 deficiency? | Recurrent of Disseminated Mycobacterial infections |
| What and when is IL-12 produced? | Produced by Macrophages soon after phagocytosis of mycobacteria |
| IL-12: | Activates T cells and NK cells, which produce in turn, INF-gamma |
| What is secreted by IL-12 activated T/NK cells? | INF-gamma |
| Mild form of M. Leprae infection: | Associated to TH1 response by CD8+ T Cells which cause the secretion of HIGH levels IL-2 and INF-gamma, leading to activation of macrophages --> mycobacterium leprae death |
| The severe form of Leprae (Leprosy)? | Humoral (TH2) response by CD4+ T cells, and release LOW levels of IL-2 and INF-gamma |
| TH-2 stimulation lead to high levels of: | IL-4, IL-5, IL-10 and IL-12 |
| What cells produce TGF-beta? | T-regulatory cells |
| What are the 3 small vessel vasculitis (+) for ANCA? | 1. MPA (Microscopic Polyangiitis) 2. Wegener's granulomatosis (GPA) 3. Churg-Strauss Syndrome ( Eosinophilic granulomatosis with polyangiitis) |
| What (+)ANCA vasculitis are associated with the presence of granulomas? | Churg-Strauss syndrome and Wegener's granulomatosis |
| Granulomatosis with polyangiitis + Eosinophilia. Dx? | Churg-Strauss syndrome |
| What are the small vessel vasculitis (-) ANCA? | 1. HSP 2. Serum Cryoglobulinemia |
| IgA dominant vasculitis + (-) ANCA. Dx? | HSP |
| What is Langerhans Cell Histiocytosis? | Disorder characterized by monoclonal proliferation of cells derived from myeloid progenitor cells, which resemble Langerhans cells found in the dermis and submucosa. |
| Where are Langerhans cells found? | Dermis and Submucosa |
| Histiocyte is another way to call a ______________________. | Macrophage |
| Patient present with fever + Rash + Bone swelling + Birbeck granules on PBS. Dx? | Langerhans cell Histiocytosis |
| What are LIVER macrophages called? | Kupffer cells |
| What are LUNG macrophages called? | Alveolar macrophages |
| What are the SKIN macrophages called? | Langerhan cells |
| Dendritic cells are: | Macrophages of the skin and Lymph nodes |
| What area of small intestine are Peyer's patches most commonly found? | Ileum |
| What substance is secreted by Peyer's patches? | IgA antibodies |
| What is the role of M cells of the Peyer's patches? | Take up antigen form the intestinal tract and present it to the B-cells. |
| Once the M-cells of Peyer's patches have presented antigen to B-cells, what happens? | B-cells secrete IgA antibodies and differentiate into plasma cells |
| What is a common cause for development of Intussusception? | GI infection of the small bowel |
| What is the clinical presentation of Intussusception? | Episodic abdominal pain, bloody diaper/stool, and recent diarrheal illness. |
| What is the most common cancer in children? | ALL |
| What is a common accompanied WBC deficit seen in patients with any Lymphoblastic Leukemia? | Neutropenia |
| Why do patients with a Lymphoblastic Leukemia often develop Neutropenia? | The excessively rapid production of WBC lead to a decrease production of other cells, leading to Bone marrow failure. |
| What is common description of an individual with IL-12 deficiency? | Chronic Respiratory infections, positive PPD test, and infection by mycobacteria. |
| IL-12 is essential for: | Cell-mediated Immune response |
| IL-12 relation with CD4+ T cells is: | IL-12 induces CD4+ T cell differentiation into TH-1 cells, which then secrete INF-gamma to activate macrophages |
| What is the treatment for IL-12 deficiency? | INF-gamma |
| What is the INF used for the tx of Multiple Sclerosis? | INF-beta |
| IgG oligoclonal bands in CSF. Dx? | Multiple Sclerosis |
| Treatment of Chronic Granulomatous Disease? | INF-gamma |
| INF-beta is common treatment of: | 1. Hepatitis B and C 2. Kaposi Sarcoma 3. Malignant Melanoma 4. Certain leukemias |
| What interleukin is used to treat Thrombocytopenia? | IL-11 |
| IL-6 inhibitors are used in the tx of : | Rheumatoid arthritis |
| What is the pathophysiology of Anaphylactic shock? | The cross-link of IgE on immunoglobulin surface receptors on both Mast cells and Basophils lead to the secretion of vasoactive compounds, leading to anaphylactic shock |
| What cells are involved in anaphylactic shock most commonly? | Mast cells and Basophils |
| What immunoglobulin is involved or associated in Anaphylactic shock development? | IgE |
| What is the role of INF-gamma? | Immunity against viruses and bacteria; acts to activate macrophages. |
| C5b is? | Component of MAC |
| What is an important mediator of SEPTIC shock? | Nitric oxide |
| What is the common name for factor 12? | Hageman Factor |
| What is the function or role of the Hageman Factor? | Activation of the Intrinsic Pathway of Coagulation |
| IL-1 causes _______________. | Fever |
| HLA-A3 is associated to: | Hemochromatosis |
| HLA-B27 is seen in: | Seronegative spondyloarthritis |
| HLA-DQ2 and HLA-DQ8 are markers for: | Celiac Disease |
| HLA-DR2 is seen with: | Multiple Sclerosis, Hay fever, and Goodpasture syndrome |
| What HLA is seen with Goodpasture Syndrome? | HLA-DR2 |
| What HLA is shared by Goodpasture syndrome and MS? | HLA-DR2 |
| What HLAs are associated with DM type 2? | HLA-DR3 and HLA-DR4 |
| What chromosome is in control of the HLA system? | Chromosome 6 |
| What is the "Complement" system? | System of hepatically synthesized plasma proteins that play a role in innate immunity and inflammation |
| The MAC defends against: | Gram negative bacteria |
| C3b -----> | Opsonization |
| C3a, C4a, and C5a are complement substances associated with: | Anaphylaxis |
| C5a is a _____________________________. | Neutrophil chemotactic |
| C5b-C9 functions to: | Cytolysis by MAC |
| IL-8 is: | Major neutrophil chemotactic |
| What is the role of Ig-3? | stimulate the proliferation of cells in the myeloid lineage |
| What substance has a similar role of Ig-3? | GM-CSF |
| Where are Birbeck granules located? | In Langerhan cells (skin APCs) |
| Birbeck granules: | Express MHC-2, which binds the CD4+ T cells, producing the first signal for helper T-cell activation. |
| Abbreviation of Warm-Reactive Hemolytic anemia | AIHA |
| What are the clinical manifestations of AIHA? | 1. Signs of anemia 2. Complications of hemolysis (jaundice due to increased indirect bilirubin) 3. Increased Reticulocyte count 4. Positive warm-agglutinin test |
| What kinds of atypical RBCs are seen in PBS of AIHA? | Schistocytes |
| What is the best initial treatment of AIHA? | Corticosteroids |
| CD-20 is a marker for: | B-cells; Involved in hyperacute rejection and humoral type acute rejection |
| CD-27: | Marker of PLASMA CELL |
| CD-34: | marker of FIBROCYTES |
| CD-4: | marker for HELPER T-CELLS |
| CD-56: | marker of Natural Killer cells |
| CD-8: | marker of cytotoxic T-cells |
| What CD markers are involved in Hyperacute rejection? | CD20, CD27, CD56 |
| What are the CD markers and cells involved in Acute rejection? | CD20 (B-cell), CD8 (cytotoxic T-cell) |
| What are the CD makers and cells involved in Chronic rejection? | CD34 (Fibrocytes), CD4 (helper T-cell) |
| What is the result or effect of CD8+ cytotoxic cells in Acute rejection? | Cytotoxic graft cell death and release of proinflammatory cytokines |
| What antibodies are involved in the protection against Influenza virus infection? | Antibodies against hemagglutinin antigen |
| How is influenza virus protection achieved if there was no annual vaccination on a patient? | The production of antibodies against hemagglutinin antigen may be induced by prior similar strain infection or past vaccination. |
| Genetic Shifts and Drifts often involve: | Large population, causing pandemics or endemic diseases |
| The antibodies in Lambert-Eaton Disease are directed to: | PREsynaptic voltage-gated CALCIUM channels |
| Repetitive nerve and/or muscle stimulation in Lambert-Eaton Disease will cause? | Worsening of symptoms |
| The alleviation or bettering of symptoms in Myasthenia Gravis is achieved by: | Repetitive and constant stimulation of Nerves and muscles |
| Which receptors are affected by antibodies in Myasthenia Gravis? | POSTsynaptic ACETYLCHOLINE receptors |
Created by:
rakomi
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